Wegener?s granulomatosis is a small vessel vasculitis, associated with various clinical manifestations, among which the most common are respiratory tract disease and glomerulonephritis leading to renal failure. Pathogenesis of vascular injury in Wegener?s granulomatosis is ascribed to antineutrophil cytoplasmic antibodies directed (ANCA) mainly against proteinase 3, an enzyme from neutrophil granules. The reasons for breakdown of self-tolerance to proteinase 3 are unknown and together with molecular mechanisms underlying this immunoinflammation are the subject of research. Standard treatment of Wegener?s granulomatosis consists of cyclophosphamide and corticosteroids. In patients resistant to that therapy or with the refractory disease some alternative strategies involving tumor necrosis factor blocade, polyclonal antithymocyte globulin or monoclonal anti-T cell antibodies are applied.